New anatomical criteria of the above malformation are created on the basis of the study of 63 hearts with the origin of aorta and lung artery from the right ventricle: the presence of the bulboventricular fold separating the mitral and semilunar valves; complete or partial delay of the subaortal cone movement to the left ventricle; the presence of the completely or partially formed proximal cone; underdevelopment of the left ventricle due to the complete or partial absence of its cone region; the only issue from the left ventricle is the proximal cone apertures or a part of the primary bulboventricular orifice; when the primary bulboventricular orifice is obturated the left ventricle is either deprived of the opening or has it in the form of noncommitted defect in the sinusal part of the interventricular septum; the anterior margins of the bulboventricular fold and cone septum are open and supraventricular comb is not formed; the interventricular septum defect formed due to failure of fusion between bulboventricular fold and cone septum connects the aorta with the right ventricle; aorta and lung artery start completely or partially from the right ventricle.